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Peer-reviewedCC BYSurgical case reports

Laparoscopic Distal Pancreatectomy with Splenectomy for a Multiloculated Primary Splenic Epidermoid Cyst Mimicking a Lymphangioma in an Older Woman: A Case Report.

5 affiliations
  1. 1Department of Pathology and Matrix Biology, Mie University Graduate School of Medicine, Tsu, Mie, Japan.
  2. 2Department of Genomic Medicine, Mie University Hospital, Tsu, Mie, Japan.
  3. 3Pathology Division, Mie University Hospital, Tsu, Mie, Japan.
  4. 4Department of Pathology and Laboratory Medicine, JA Suzuka General Hospital, Suzuka, Mie, Japan.
  5. 5Department of Surgery, Suzuka Kaisei Hospital, Suzuka, Mie, Japan.

Abstract

Introduction

Primary splenic epidermoid cysts typically occur in young adults as unilocular lesions and are usually distinguished from lymphangiomas on histopathology, although preoperative imaging differentiation remains challenging. A multiloculated presentation in an older patient is uncommon and can be mistaken for a lymphangioma.Case presentation

Herein, we report a 75-year-old asymptomatic woman with a multiloculated splenic cyst that was managed conservatively for approximately 10 years. CT and MRI favored a lymphangioma, and surgical resection was performed because of interval growth. Laparoscopic distal pancreatectomy with splenectomy (DP-S) was required because the cyst was firmly adherent to the pancreas. Histopathological examination revealed a 3.7 × 3.1-cm multiloculated cyst within the splenic capsule, lined by stratified squamous epithelium and associated with foamy histiocytes and cholesterol crystal clefts. Immunohistochemistry demonstrated negativity for CD31, CD34, and D2-40, and positivity for AE1/AE3, cytokeratin (CK) 5/6, and CK7, confirming a primary splenic epidermoid cyst. Negativity for calretinin and Wilms tumor 1 (WT-1) excluded a mesothelial cyst, whereas basal p63 positivity and carbohydrate antigen 19-9 expression supported the multilayered subtype. A postoperative pancreatic fistula was managed conservatively, and the patient was discharged on POD 34.Conclusions

Integrated clinicopathological assessment, rather than any single marker, is needed to distinguish a multiloculated splenic epidermoid cyst from a lymphangioma; comprehensive immunohistochemistry, including D2-40, calretinin, WT-1, and p63, is a useful adjunct. This case also shows that a benign splenic cyst may require distal pancreatectomy: when dense pancreatic adhesion is encountered intraoperatively, en bloc DP-S can become an unavoidable step toward safe and complete resection, and postoperative pancreatic fistula remains a relevant complication to anticipate.

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BibTeX
@article{hashizume2026laparoscopic,
  title = {{Laparoscopic Distal Pancreatectomy with Splenectomy for a Multiloculated Primary Splenic Epidermoid Cyst Mimicking a Lymphangioma in an Older Woman: A Case Report.}},
  author = {Ryotaro Hashizume and Hiroshi Imai and Tetsuya Murata and Yasuo Okura},
  journal = {Surgical case reports},
  year = {2026},
  month = oct,
  doi = {10.70352/scrj.cr.26-0466},
  url = {https://europepmc.org/article/MED/42936885},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Laparoscopic Distal Pancreatectomy with Splenectomy for a Multiloculated Primary Splenic Epidermoid Cyst Mimicking a Lymphangioma in an Older Woman: A Case Report.
AU  - Ryotaro Hashizume
AU  - Hiroshi Imai
AU  - Tetsuya Murata
AU  - Yasuo Okura
PY  - 2026
DA  - 2026/10/24
JO  - Surgical case reports
DO  - 10.70352/scrj.cr.26-0466
UR  - https://europepmc.org/article/MED/42936885
AB  - Introduction  Primary splenic epidermoid cysts typically occur in young adults as unilocular lesions and are usually distinguished from lymphangiomas on histopathology, although preoperative imaging differentiation remains challenging. A multiloculated presentation in an older patient is uncommon and can be mistaken for a lymphangioma.Case presentation  Herein, we report a 75-year-old asymptomatic woman with a multiloculated splenic cyst that was managed conservatively for approximately 10 years. CT and MRI favored a lymphangioma, and surgical resection was performed because of interval growth. Laparoscopic distal pancreatectomy with splenectomy (DP-S) was required because the cyst was firmly adherent to the pancreas. Histopathological examination revealed a 3.7 × 3.1-cm multiloculated cyst within the splenic capsule, lined by stratified squamous epithelium and associated with foamy histiocytes and cholesterol crystal clefts. Immunohistochemistry demonstrated negativity for CD31, CD34, and D2-40, and positivity for AE1/AE3, cytokeratin (CK) 5/6, and CK7, confirming a primary splenic epidermoid cyst. Negativity for calretinin and Wilms tumor 1 (WT-1) excluded a mesothelial cyst, whereas basal p63 positivity and carbohydrate antigen 19-9 expression supported the multilayered subtype. A postoperative pancreatic fistula was managed conservatively, and the patient was discharged on POD 34.Conclusions  Integrated clinicopathological assessment, rather than any single marker, is needed to distinguish a multiloculated splenic epidermoid cyst from a lymphangioma; comprehensive immunohistochemistry, including D2-40, calretinin, WT-1, and p63, is a useful adjunct. This case also shows that a benign splenic cyst may require distal pancreatectomy: when dense pancreatic adhesion is encountered intraoperatively, en bloc DP-S can become an unavoidable step toward safe and complete resection, and postoperative pancreatic fistula remains a relevant complication to anticipate.
SN  - 2198-7793
ER  -